Immunoglobulin G4 sinusitis in association with aspirin-exacerbated respiratory disease

Immunoglobulin G4 (IgG4) related disease is a systemic inflammatory disease characterized by tumor-like tissue infiltration with IgG4 positive (IgG4+) plasma cells. Aspirin-exacerbated respiratory disease (AERD) is defined as asthma, chronic rhinosinusitis with nasal polyposis, and hypersensitivity to cyclooxygenase-1 inhibitors.

We described a case of a non-smoking 61-year-old male with prior NSAID sensitivity who presented with a 1-year history of left eye proptosis associated with chronic nasal symptoms, ultimately identified as concurrent AERD and IgG4 sinusitis.

The patient was evaluated in the clinic and diagnosed by using clinical, radiographic, and surgical biopsy findings.

Although initial concern was greatest for malignancy, a biopsy specimen confirmed the presence of a dense lymphoplasmacytic infiltrate and storiform fibrosis, associated with increased IgG4+ plasma cells. Therefore, IgG4-related disease (RD) was identified in this patient with AERD.

Shared type II inflammation may be responsible for the coexistence of IgG4-RD and AERD as observed in our patient. Health care workers must be cognizant of the simultaneous presentation of both IgG4-RD and AERD.